Vol.47 - Número 3, Julio/Septiembre 2018 Imprimir sólo la columna central

Sudden death by Stanford type-A aortic dissection in a 10-year-old child

SOLANGEL GUTIERREZ EGUES, OMAIDA J. LÓPEZ BERNA,
LEIDELÉN ESQUIVEL SOSA
Hospital Pediátrico José Luis Miranda.
Rpto Ramón Ruiz del Sol. Villa Clara, Cuba
E-mail
Recibido 21-JUN-2018 – ACEPTADO despues de revisión el 14-JUL-2018.
There are no conflicts of interest to disclose.

 

Male, 10-year-old patient, with history of apparent health and first symptoms of oppressive, sharp central thoracic pain. He was provided care in his neighborhood, where he died suddenly. He was then moved to the Pediatric Hospital to determine cause of death by necropsy.

The macroscopic test showed enlarged pericardial sac, with blood content of 400 cc. Mildly enlarged heart. In its opening, where the aorta emerges, at 2 cm from the valve, a 4 cm oblique tear was observed, separating the tunica intima from the tunica media, with large bruise dissecting the proximal portion of the ascending aorta until the emergence of the brachiocephalic trunk, flooding the pericardial sac (Stanford type-A aortic dissection, DeBakey type II).

Microscopically, cystic degeneration manifested in the tunica media.

Aortic dissection produces a false lumen in the tunica media of the aortic wall, which is exceptional in children with no connective tissue disease [1].

Some authors prefer using the Stanford classification currently [2], rather than the DeBakey one, as it is considered to be more practical.

Transesophageal echo is considered the initial study due to its availability, harmlessness, and low cost, besides allowing to locate the intima tear among other complications. Multislice computed tomography has a high sensitivity and specificity due to the reconstructions enabled by it. Nuclear magnetic resonance defines ventricular volumes, functional valve alterations and dynamic behavior of the aortic wall [3].

Anatomopathologically, there is degeneration of the smooth muscles of the tunica media of the aortic wall, in type-B aortic dissection, proper of hypertensive patients and in individuals with an advanced age. Type-A aortic dissection has been associated to congenital anomalies of the conjunctive tissue at the level of the elastic tissue of the aortic tunica media, frequently in young people, and very frequent in Marfan syndrome. The Ehler-Danlos, Loeys-Dietz, and Turner syndromes predispose it [4].

Aortic rupture is one of the main causes of premature death in patients with acute and subacute Stanford type-A aortic dissection. The shock, the pain requiring medications, a troponin level >0.7 ng/ml and a dimer level D≥10 µg/ml are independent risk factors for aortic rupture in these patients [5].

The risk of sudden cardiac death or aortic dissection is still low in patients with Marfan syndrome and aortic diameter between 45 and 49 mm. An aortic diameter ≥50 mm seems to be a reasonable threshold for preventive surgery [6].

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BIBLIOGRAPHY

  1. Serrrano Olmedo SC, Espinoza Saquicela ER. Ascending aortic disease in a patient with Marfan Syndrome. Case report 2017; 3 (2): 98-106, ISSN electrónico 2462-8522.
  2. Bustamante-Munguira J, Juez M. Síndrome aórtico agudo. Cir Cardiov 2016; 23 (1): 38-44.
  3. Evangelista A, Rabasa JM, Mosquera VX, et al. Diagnosis, management and mortality in acute aortic syndrome: results of the Spanish Registry of Acute Aortic Syndrome (RESA-II). Eur Heart J Acute Cardiovasc Care 2016;  Dec 1:2048872616682343. doi: 10.1177/2048872616682343
  4. Wang TC, Kuo CL. Aortic Intramural Hematoma, Stanford Type A. J Emerg Med 2017; 53 (6): e137-e138.
  5. Li ZD, Liu Y, Zhu J, et al. Risk factors of pre-operational aortic rupture in acute and subacute Stanford type A aortic dissection patients. J Thorac Dis 2017; 9 (12): 4979-87.
  6. Jondeau G, Detaint D, Tubach F, et al. Aortic event rate in the Marfan population: a cohort study. Circulation 2012; 125 (2): 226-32.

Publication: September 2018



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